Carlos Ewerton Maia Rodrigues

Neurological manifestations of antiphospholipid syndrome

Eur J Clin Invest 2010; 40 (4): 350–359

Pediatric catastrophic antiphospholipid syndrome: Descriptive analysis of 45 patients from the “CAPS Registry”

Given the lack of information about catastrophic antiphospholipid syndrome (APS) in pediatric patients, the objective of the current study was to describe the clinical characteristics, laboratory features, treatment, and outcome of pediatric patients with catastrophic APS and compare them with the adult patients with catastrophic APS. We identified patients who were under 18years of age at time of catastrophic APS diagnosis included in the international registry of patients with catastrophic APS (CAPS Registry). Their main demographic and clinical characteristics, laboratory features, treatment, and outcome were described and compared with those of adult patients with catastrophic APS. From the 446 patients included in the CAPS Registry as of May 2013, 45 (10.3%) patients developed 46 catastrophic events before 18years of age (one patient presented two episodes). Overall, 32 (71.1%) patients were female and the mean age was 11.5±4.6years (range, 3months-18years). A total of 31 (68.9%) patients suffered from primary APS and 13 (28.9%) from systemic lupus erythematosus (SLE). The main differences between the two groups of patients were the higher prevalence of infections as precipitating factor for catastrophic event in the pediatric population (60.9% versus 26.8% in the adult population, p<0.001) and of peripheral vessel thrombosis (52.2% versus 34.3%, p=0.017). In addition, catastrophic APS was the first manifestation of APS more frequently in pediatric patients (86.6% versus 45.2%, p<0.001). Interestingly, pediatric patients showed a trend of lower mortality, although the difference was not statistically significant (26.1% versus 40.2%; odds ratio, 1.9; 95% confidence interval, 0.96-3.79; p=0.063). No differences were found neither in the laboratory features nor in the isolated or combination treatments between groups. Catastrophic APS in pediatric patients is a rare disease. There are minimal differences in the clinical and laboratory features, treatment, and outcome of pediatric and adult catastrophic APS patients.

Clinical, radiologic, and therapeutic analysis of 14 patients with transverse myelitis associated with antiphospholipid syndrome: report of 4 cases and review of the literature.

OBJECTIVE To analyze the clinical, radiologic, therapeutic, and developmental characteristics of transverse myelitis (TM) and antiphospholipid syndrome (APS). METHODS We systematically searched English, Spanish, and Japanese articles on the subjects of TM and APS that had English abstracts in PubMed from 1966 to 2010. In addition, we reported on 4 patients with APS and TM that were treated by the Rheumatology Division of the Hospital das Clínicas da Faculdade de Medicina da Universidade in São Paulo, Brazil. RESULTS Fourteen cases of patients with APS and TM were reviewed. The age of these patients ranged from 8 to 83, and cases of TM predominantly occurred among patients with primary APS (9/14). The clinical presentation of TM was characterized by effects on the thoracic spinal cord (9/14) that were associated with sphincter disturbances (8/14). The onset of symptoms was sudden in 8/14 cases, and the symptoms of myelitis were recurring in 3 cases. One case resulted in death. In most cases, treatment was based on corticosteroid pulse therapy (12/14), but some patients were treated with pulse cyclophosphamide (5/14), plasmapheresis (3/14), or rituximab (1/14). Generally, the therapeutic response was satisfactory, and complete improvement was seen in 9/14 patients. CONCLUSION In light of the severe clinical presentation of TM and its morbidity and mortality, early diagnosis and aggressive treatment are vital for therapeutic success. We can verify the excellent therapeutic response, as we saw a complete improvement in 64% of patients.

Clinical, Laboratory, and Therapeutic Analyses of 21 Patients with Neonatal Thrombosis and Antiphospholipid Antibodies: A Literature Review

Objectives. A review of the literature reports neonatal thrombosis and antiphospholipid antibodies cases through a retrospective study that focuses on the pathogenesis and main clinical and laboratory manifestations of this disease. Methods. The case reports were selected from PubMed. The keywords used to search were neonatal, antiphospholipid syndrome, thrombosis, and antiphospholipid antibodies. References that were published from 1987 to 2013 were reviewed. Results. Twenty-one cases of neonatal thrombosis and antiphospholipid antibodies were identified. Ten children were born preterm (before 37 weeks). Arterial involvement (17/21) was predominant, of which stroke (12/17) was the most prevalent clinical manifestation. Anti-cardiolipin antibodies were predominant (13/21) in the antiphospholipid antibody profiles. Treatments were based on the use of symptomatics such as antiepileptics (8/21), and 6/21 patients received heparin. There were 4 deaths (4/21); otherwise, the children recovered well, especially the neonates who suffered from strokes (9/12). Conclusion. Neonatal thrombosis and antiphospholipid antibodies are rare. The development of thrombotic manifestations in neonates seems not to be associated exclusively with the aPL, but their etiology may be linked to pre- and perinatal events. We noted good therapeutic responses, especially in stroke patients, who presented with favorable outcomes in 82% of the cases.

Association of arterial events with the coexistence of metabolic syndrome and primary antiphospholipid syndrome

Metabolic syndrome (MetS) is highly prevalent in rheumatic diseases and is recognized as a new independent cardiovascular risk factor. This study was undertaken to determine the clinical significance of MetS in patients with primary antiphospholipid syndrome (APS).

Diffuse systemic sclerosis and autoimmune hepatitis: a unique association

Autoimmune hepatitis (AIH) is a chronic hepatitis of unknown etiology characterized by continuing hepatocellular necrosis and inflammation that afflicts 100,000 to 200,000 persons in the United States. It is a rare manifestation of systemic sclerosis. Only about nine reports of this association have been previously reported in the literature. Importantly, all cases had the limited clinical form of systemic. The authors describe herein the first report of a patient with diffuse systemic sclerosis who was diagnosed with AIH with positive antimitochondrial antibody and had an excellent response to immunosuppressive drugs. We also briefly review the literature regarding this issue.

Review on anti-lipoprotein lipase antibodies

Lipoprotein lipase (LPL) is a member of the lipase family, and LPL is known to hydrolyze triglyceride molecules found in lipoprotein particles. Understanding the pathogenesis of atherosclerosis in autoimmune diseases, particularly systemic lupus erythematosus (SLE), is vital to reduce morbidity and mortality of this disease. It is now recognized that SLE has a particular pattern of dyslipoproteinemia characterized by low HDL levels and increased triglycerides, which is aggravated by flare. Antilipoprotein lipase (anti-LPL) antibodies have been recently described in rheumatic diseases, mainly in SLE and systemic sclerosis (SSc). Several studies have reported a close link between antibodies, inflammation and lipoprotein levels and an increased risk of cardiovascular damage in this particular group of patients. These studies have emphasized the importance of rigorous clinical control of disease activity and prevention of cardiovascular risk factors. This review summarized the studies that have discussed the presence of anti-LPL in rheumatic diseases and other conditions and analyzed the importance of this antibody in the complex atherosclerotic process in autoimmune diseases.

Heparin Increases HLA-G Levels in Primary Antiphospholipid Syndrome

Objectives. The aim of this study was to investigate the HLA-G serum levels in Primary Antiphospholipid Syndrome (PAPS) patients, its impact on clinical and laboratory findings, and heparin treatment. Methods. Forty-four PAPS patients were age and gender matched with 43 controls. HLA-G serum levels were measured using an enzyme-linked immunosorbent assay (ELISA). Results. An increase in soluble HLA-G levels was found in patients compared to controls (3.35 (0–22.9) versus 1.1 (0–14), P = 0.017). There were no significant differences in HLA-G levels between patients with and without obstetric events, arterial thrombosis, venous thrombosis, or stroke. Sixty-six percent of patients were being treated with heparin. Interestingly, patients treated with heparin had higher HLA-G levels than ones who were not treated with this medication (5 (0–22.9) versus 1.8 (0–16) ng/mL, P = 0.038). Furthermore, patients on heparin who experienced obstetric events had a trend to increased HLA-G levels compared to patients who were not on heparin and did not have obstetric events (5.8 (0–22.9) versus 2 (0–15.2) ng/mL, P = 0.05). Conclusion. This is the first study to demonstrate that serum HLA-G levels are increased in APS patients. We also demonstrated that heparin increases HLA-G levels and may increase tolerance towards autoantigens.

Sleep disorders in primary antiphospholipid syndrome

The objective of this study was to evaluate the sleep quality, the presence of sleep disorders in patients with primary antiphospholipid syndrome (pAPS), and their possible clinical and laboratory associations. This was a cross-sectional study of 40 consecutive pAPS patients and 211 healthy age- and sex-matched controls. Demographic and clinical data, drug use, and antiphospholipid antibodies were evaluated. Sleep was evaluated using the Pittsburgh Sleep Quality Index (PSQI). pAPS patients had significantly worse sleep quality than healthy controls. Analyzing the individual components, pAPS had worse scores in five of seven components: sleep duration (p = 0.002), habitual sleep efficiency (p = 0.003), sleep disturbance (p < 0.001), use of sleep medication (p < 0.001), and daytime somnolence (p = 0.03). No association of sleep disturbance and demographic, clinical, and laboratory features of the disease was observed. This is the first study to analyze sleep quality in pAPS. We observed that pAPS had significant worse sleep quality; however, no demographic, clinical, or laboratory feature was associated with sleep disturbance.

IgA deficiency in primary antiphospholipid syndrome.

Joint Bone Spine - In Press.Proof corrected by the author Available online since mercredi 26 juin 2013